The New England Journal of MedicineAL Amyloidosis

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This “Images in Medicine” report illustrates a 58-year-old man with AL amyloidosis associated with lambda light-chain myeloma. The patient presented with distinctive eyelid lesions, periorbital petechiae, and systemic symptoms. Diagnostic procedures, including biopsy and histopathological testing, revealed characteristic findings of amyloidosis. The case highlights the importance of recognizing clinical signs and conducting appropriate tests for accurate diagnosis and treatment of this rare condition.

Key Points:

• Patient presented with a 2-year history of eyelid lesions, weight loss, and fatigue
• Physical examination revealed periorbital petechiae, purpura, waxy eyelid papules, and macroglossia
• Biopsy and histopathological testing showed amyloid deposits with positive Congo red staining and apple-green birefringence
• Direct immunofluorescence demonstrated predominant lambda light-chain deposition
• Additional tests confirmed AL amyloidosis associated with lambda light-chain myeloma
• Treatment initiated with daratumumab, bortezomib, cyclophosphamide, and dexamethasone
• Partial response observed after three cycles; autologous stem-cell transplantation planned

In the United States, AL amyloidosis is the most common type, with approximately 4,500 new cases diagnosed every year. It usually affects people from ages 50-80, although there are a few cases of people being diagnosed as early as their late 20s. About two-thirds of the patients are male. (Amyloidosis Foundation)

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